The Internet Encyclopedia of Science has gathered what may be the most compact, yet comprehensive chunk of helpful info on congenital heart defects ever assembled.  It’s easy to read, easy to understand and provides a launching pad for those new to pediatric heart disease.  Know more.  Do more.

congenital heart defects

• Slow down
• Go in the wrong direction or to the wrong place
• Be blocked completely

Congenital heart defect is the most common type of major birth defect. Each year, more than 40,000 babies in the United States are born with congenital heart defects.

Types of congenital heart defects

There are many types of congenital heart defects. They include:

• Abnormal passages in the heart or between blood vessels
• Problems with the heart valves
• Problems with the placement or development of blood vessels near the heart
• Problems with development of the heart itself

Some of these problems are described below.

Abnormal passages in the heart or between blood vessels

• Atrial septal defect (ASD) is a hole in the wall that separates the upper chambers, or atria, of the heart. This causes blood to leak from one atrium to the other.
• Ventricular septal defect (VSD) is a hole in the wall that separates the lower chambers, or ventricles, of the heart. This causes blood to leak from one ventricle to the other.
• Atrioventricular septal defect (AVSD) includes an ASD, VSD, and abnormal development of the atrioventricular valves (tricuspid and mitral). This causes blood to flow abnormally inside the heart. An AVSD is also known as an atrioventricular canal defect.
• Patent ductus arteriosus (PDA) is a persistent connection between the aorta and the pulmonary artery. This connection is called the ductus arteriosus and is normally present before birth. In most babies, the vessel closes within a few hours or days after birth. In some children, the vessel fails to close, resulting in PDA.

Problems with the heart valves

Congenital heart defects can involve any of the valves and include the following types of problems:

• Stenosis. The valve opening is narrow and does not open completely.
• Atresia. The valve does not form, so there is no opening for blood to pass from one chamber to another.
• Regurgitation. The valve does not close completely, so blood can leak back through the valve.

Examples of particular heart valve problems include:

• Aortic valve stenosis is a narrowing of the aortic valve in the heart that causes it to open incompletely. This can reduce blood flow to the body.
• Pulmonary valve atresia is a defect in which a solid sheet of tissue forms in place of the pulmonary valve. This prevents blood in the right side of the heart from traveling normally to the lungs to pick up oxygen.
• Pulmonary valve stenosis is a narrowing of the pulmonary valve. The narrowing slows the flow of blood from the right side of the heart to the lungs. The heart must pump harder to push blood through the smaller opening.
• Tricuspid valve atresia is a defect in which a solid sheet of tissue forms in place of the tricuspid valve. Without the tricuspid valve, blood entering the right atrium cannot travel normally to the right ventricle and then to the lungs to pick up oxygen.
• Ebstein’s anomaly is a defect in which the tricuspid valve is both displaced and abnormally formed. The valve leaks and allows blood to flow back into the right atrium instead of to the lungs to pick up oxygen.

Problems with placement or development of blood vessels near the heart

• Transposition of the great vessels is a defect in which the location of the “great vessels” (the aorta and pulmonary artery) coming off the heart is switched. The aorta comes off the right ventricle instead of the left ventricle. The pulmonary artery comes off the left ventricle instead of the right ventricle. Therefore, blood without oxygen is continually pumped to the body, instead of blood with oxygen.
• Tetralogy of Fallot is a combination of four defects:
  • Pulmonary valve stenosis is the narrowing of the pulmonary valve. The narrowing slows the flow of blood from the right ventricle to the lungs.
  • VSD is a hole in the wall that separates the left and right ventricles.
  • Overriding aorta is a defect in which the aorta is positioned between the left and right ventricles, over the VSD.
  • Right ventricular hypertrophy is the thickening of the right ventricle. The thickening is caused by the heart having to work harder because of the other defects.
• Truncus arteriosus is a defect of the great vessels. The aorta and pulmonary artery do not form as separate arteries. Instead, a large artery, called the truncus, comes from the heart. As the truncus leaves the heart, it may branch into arteries that carry blood to the body and to the lungs.
• Coarctation of the aorta is a narrowing of the aorta. It slows or blocks the flow of blood from the heart to the body.
• Anomalous pulmonary venous return is a defect in which one or more of the four pulmonary veins, which normally return oxygen-rich blood from the lungs to the heart, return to the wrong chamber in the heart.

Problems with development of the heart

• Hypoplastic left heart syndrome is a combination of defects in which the left side of the heart does not develop properly. Defects usually include mitral atresia, aortic atresia, and a tiny left ventricle.
• Mitral atresia occurs when a solid sheet of tissue forms instead of the mitral valve, which separates the left atrium and the left ventricle.
• Aortic atresia occurs when a solid sheet of tissue forms instead of the aortic valve, which separates the left ventricle from the aorta.
• Single ventricle describes a group of heart defects in which only one ventricle is present instead of two. It can be a single right or a single left ventricle. The other ventricle is usually absent or very tiny. Hypoplastic left heart syndrome is an example of a single ventricle defect.

Today, the outlook for an infant born with a heart defect is much better than it was 30 years ago. Rapid advances in infant and childhood surgery, better tests, and new medicines help most children with congenital heart defects. Many children born with more complex or severe heart defects now reach adulthood. Today, there are more than 1 million adults living with congenital heart defects.

What causes congenital heart defects?

Doctors do not know what causes most cases of congenital heart defects. Heredity may play a role. In rare cases, more than one child in a family is born with a heart defect. Also, parents who have a congenital heart defect may be more likely than other parents to have a child with the condition.

Babies who have certain other birth defects, such as Down’s syndrome, are also more likely to have congenital heart defects.

Other factors that raise the risk for congenital heart defects are:

• Having viral infections, such as German measles (rubella), during pregnancy
• Having diabetes
• Taking some types of prescription or over-the-counter medicines during pregnancy
• Being repeatedly exposed to some chemicals or X-rays during pregnancy
• Using alcohol or street drugs during pregnancy

Research continues to find the causes of congenital heart defects.

Signs and symptoms of congenital heart defects

The most common signs and symptoms of congenital heart defects are:

• A heart murmur
• A bluish tint to skin, lips, and fingernails (“blue baby”)
• Fast breathing
• Shortness of breath
• Poor feeding, especially in infants because they tire easily while nursing
• Poor weight gain in infants
• Tiring easily during exercise or activity (older children)

The signs and symptoms that a child has depend on:

• The number and types of defects
• The severity of the defect

Some infants and children have no signs or symptoms. Others have severe or life-threatening symptoms.

Many types of congenital heart defects cause the heart to work harder than it should. This stresses the heart and can lead to heart failure, causing the heart muscle to weaken and the heart to enlarge.

Diagnosis

Doctors usually diagnose congenital heart defects during pregnancy or within the first few months after birth. Some children with less severe defects are not diagnosed until they are older and more demands are put on their hearts. Others are not diagnosed until they are adults.

If your child’s doctor suspects a congenital heart defect, he or she will refer your child to a specialist who treats heart problems in children. The specialist, a pediatric cardiologist, will take a family and medical history, do a physical exam, and order several tests.

Physical exam

During the physical exam, the doctor:

• Listens to your child’s heart with a stethoscope for a heart murmur
• Looks for signs of illness or physical problems, such as bluish color of skin and lips, shortness of breath, rapid breathing, and delayed growth Listens to your child’s lungs

Tests

An echocardiogram, which is harmless and painless, uses sound waves to create a moving picture of your child’s heart. During an echocardiogram, reflected sound waves outline the heart’s structure completely. The test allows the doctor to clearly see any problem with the way the heart is formed or the way it’s working. An echocardiogram is the most important test available to your child’s cardiologist to both diagnose a heart problem and follow the problem over time. In children with congenital heart defects, an echocardiogram will outline each problem with the heart’s structure and show how the heart is reacting to these problems. The echocardiogram will help your child’s cardiologist to know if and when treatment is needed.

During pregnancy, if your doctor suspects that your baby has a congenital heart defect, a special test called a fetal echocardiogram can be done. This test uses sound waves to create a picture of the baby’s heart while the baby is still in the womb. The test is usually done during the fifth month of pregnancy. If your child is diagnosed with a congenital heart defect before birth, your doctor can plan treatment before the baby is born.

Other tests used to help diagnose congenital heart defects include:

• EKG (electrocardiogram). An EKG test measures the rate and regularity of your child’s heartbeat.

• Chest X-ray. A chest X-ray takes a picture of your child’s heart and lungs. It can show if the heart is enlarged or if there is fluid in the lungs.

• Pulse oximetry. This test uses a sensor to see how well your child’s lungs are passing oxygen to the blood and whether there is any mixing of oxygen-rich and oxygen-poor blood. The sensor is placed on the child’s fingertip or toe (like an adhesive bandage). A small computer unit shows the amount of oxygen in the blood through the skin. The test does not hurt.

• Cardiac catheterization. In this test, a thin flexible tube is passed through an artery or vein at the upper thigh (groin) or in the arm to reach the heart. With the assistance of X-rays, the doctor can then see your child’s blood vessels and heart. The catheter also measures the pressure inside the heart and blood vessels and can determine if blood is mixing between the two sides of the heart. Sometimes, a dye that can be seen by X-ray is injected into the heart. This enables the doctor to see the flow of blood throughout the heart and blood vessels.

Treatment

Doctors treat congenital heart defects with:

• Medicines
• Special procedures using catheters
• Surgery
• Heart transplants

The treatment your child receives depends on the type and severity of the defect. Other factors include your child’s age, size, and general health. Treatment can be simple or very complex. Many children are treated with medicines and are monitored by their doctor. Other children may need surgery.

Medicines

Your child may take one or more of the following medicines to help the heart work better and lessen symptoms: Digoxin is thought to improve heart function and can keep the heartbeat regular. Diuretics treat the buildup of fluid in the heart and body. ACE inhibitors decrease the work the heart has to do and may help remodel the heart and blood vessels to work more efficiently. Beta blockers slow the heart rate and lower blood pressure to decrease the workload on the heart. Inotropes strengthen the heart’s pumping ability. Prostaglandin E1 is used to keep the ductus arteriosus open in some defects until corrective surgery can be carried out. This improves blood flow and oxygen levels until the defect is corrected. The ductus arteriosus normally closes within a few days after birth.

Special procedures using catheters

Doctors can correct some congenital heart defects during cardiac catheterization. These are called catheter-based procedures or interventions. They can be used instead of open-heart surgery, which is a major operation. A catheter is inserted through a blood vessel in your child’s groin. It is then threaded to the heart, where some holes in the interior walls of the heart can be fixed, a patent ductus arteriosus can be closed, and narrow valves and blood vessels can be opened up. Cardiac catheterization:

• Does not require your child’s chest to be opened
• Lets your child recover quickly
• Has different risks than open-heart surgery

Surgery

Your child may need open-heart surgery if the defect can’t be repaired using a catheter-based procedure. Some surgeries repair the defect completely. Other surgeries improve the child’s health but do not completely repair the defect. Open-heart surgery may be done to:

• Close holes with stitches or with a patch
• Repair valves
• Widen arteries or openings to valves
• Put the great vessels (aorta and pulmonary artery) in their correct positions

Sometimes, open-heart surgery can improve a child’s health but not repair the problem. Examples include:

• Decreasing blood flow to the lungs by placing a band around the pulmonary artery
• Increasing blood flow to the lungs by connecting an artery from the aorta to the pulmonary artery
• Connecting the veins that bring oxygen-poor blood directly to the pulmonary artery in a three-stage surgery when the right ventricle is not developed (for example, hypoplastic left heart syndrome)

Heart transplants

Babies born with multiple defects that are too complex to repair may need a heart transplant. In this procedure, the child’s heart is replaced with a healthy heart that has been donated.

How can congenital heart defects be prevented?

There is no known way to prevent congenital heart defects, but there are things you can do to lower the chance that your baby will have a congenital heart defect. However, even after you lower the risks, your baby may still develop a congenital heart defect.

If you are planning to become pregnant or are pregnant, talk to your doctor about any medicines that you are taking, including:

• Over-the-counter medicines
• Prescription medicines
• Vitamin and mineral supplements
• Herbal supplements

Your doctor will recommend that you take folate before you become pregnant. This is recommended mainly to prevent abnormalities in the baby’s nervous system, and there is some evidence that it may also help prevent certain types of congenital heart defects.

You should avoid:

• Strong chemicals, including some cleaning products
• Repeated exposure to X-rays
• Any harmful or poisonous materials.

If you or anyone in your family has congenital heart disease, genetic testing may be available. This may show a genetic cause for congenital heart disease. Testing cannot prevent congenital heart disease, but may be able to make you aware of the risks.

Living with a congenital heart defect

With new advances in testing and treatment, most children with congenital heart defects grow into adulthood and live productive lives. Some continue to need specialized care for survival and to maintain a good quality of life. Some may need multiple procedures, including cardiac catheterizations and surgeries. Others may need pacemakers to help their hearts beat properly.

Parents

Mothers of children born with a heart defect often think that they did something wrong during pregnancy to cause the problem. The cause of most cases of congenital heart defects is unknown. It is important that mothers know that they did not cause their child’s illness.

Most children with congenital heart defects grow up to a healthy adulthood. Only children with complex heart defects may continue to need special medical attention into adulthood.

Treatment and care for your child may be costly. The cost of surgery and hospital stays is very high. Your health insurance may not pay for everything. Some parents may need help in paying medical bills. You can get information from your doctor and hospital about how to apply for financial aid.

It is important to keep your health insurance current. If you change jobs, make sure that the new health insurance will cover your child who has a congenital heart defect. Some health insurance plans may not cover some medical conditions that you or your covered family member had before joining the new plan.

It is also very important for your child to have health insurance as adulthood approaches. Review your current health insurance plan. Find out how coverage can be extended to your child beyond the age of 18. Some policies may allow you to keep your child on your plan if he or she remains in school or is disabled.

Caring for a child with a serious heart problem can be demanding. If both parents work, one of them may have to quit and stay home with the child if suitable day care can’t be found. The drain on energy, emotions, and finances can be very stressful. Ask your child’s doctor about support groups and other types of support in your area. Seek counseling if the stress is overwhelming.

General issues

It is important for your child to have ongoing regular medical care. This includes:

• Following up with your child’s heart specialist as directed
• Following up with your child’s pediatrician or family doctor for routine exams
• Taking medicines as prescribed

You may consider having your child wear a medical alert bracelet or necklace. This tells anyone caring for your child that your child has a congenital heart defect.

Adults, teenagers, and children should have routine dental care to prevent infections of the mouth. Most people with congenital heart defects need to take antibiotics before a dental procedure, treatment, or cleaning. Talk to your doctor before going to the dentist.

Children with congenital heart defects

Some children with congenital heart defects do not grow and develop as fast as other children who are the same age. Your child may be smaller and thinner than other children. Your child may also start activities such as rolling over, sitting, and walking later than other children. After treatments and surgery, growth and development often improve.

Your child may need extra calories to grow. If your child’s heart has to pump faster because of the defect, the body needs more energy to keep up the extra work. This may cause your child to tire quickly. Some babies are not able to eat enough because they tire while feeding. Older children also may tire before finishing a meal.

Exercise for children

Exercise helps children strengthen their muscles and stay healthy. Your child may tire easily, so he or she should rest or take frequent breaks during activities, if necessary. Some children with congenital heart defects may need to limit the amount or type of exercise they do. Talk to your doctor about what is safe exercise for your child.

Remember to ask your doctor for a note for school and other organizations describing any limits on your child’s exercise or physical activities.

Emotional issues for children

Children with a serious heart problem may have a hard time coping or may feel isolated if they have to be in the hospital frequently. Some children feel sad or frustrated with their body image and their inability to be a “normal” kid. Sometimes brothers or sisters are jealous of all the attention received by the child with a congenital heart defect.

Parents may feel stress over financial and insurance issues. Your child may feel that he or she is the cause of this stress. Help your child by talking to him or her, and allow your child to be involved in everyday activities. If you have concerns about your child’s emotional health, talk to your child’s doctor.

Teenagers with congenital heart defects

The teenage years are difficult for most children. It is a time of risk taking, and denial is often used as a way of coping. There is pressure to be like other teenagers. Some teenagers with congenital heart defects still need specialized care and must take medicine on a regular basis. Help your teenager cope with the stresses of growing up with a serious health problem. Remind your teenager that, with proper care, he or she can grow up healthy and participate in most, if not all, activities.

Let your teenager help make decisions about medical care. This fosters independence and encourages regular followup with the doctor.

Many teenagers with congenital heart defects can participate in organized sports. Restrictions of activity vary depending on the teenager, the type of sport, and the type of heart defect. Some children may need to limit the amount of activity, and others may need to avoid contact sports. Your doctor can help you and your teenager decide which activities are right for him or her.

Overall, parents should be aware that the majority of children born with congenital heart defects live normal, healthy lives and should be encouraged to do so.

Adults with congenital heart defects

Today, most babies born with heart defects will live to be adults. Many of these adults believe that the surgery they had in childhood was a “cure” and may not realize that regular medical followup is needed to survive and maintain good health.

When thinking about changing jobs, adults with congenital heart defects should carefully consider the impact on their health insurance coverage. Some health plans have waiting periods or clauses to exclude some kinds of coverage. Before making any job changes, find out if the change will affect your health insurance coverage.

There are laws that protect people with congenital heart defects who are having trouble getting a job. The Americans with Disabilities Act and Work Incentives Improvement Act try to ensure equal hiring for all people, including those with health conditions.

Pregnancy and congenital heart defects

Women with congenital heart defects who want to become pregnant (or who are pregnant) should:

• Talk to their doctor about health risks during pregnancy
• Talk to their doctor about medicines that can be taken during pregnancy
• Consult with specialists who take care of pregnant women with congenital heart defects

Presentation to the Secretary’s Advisory Committee on Heritable Disorders in Newborns and Children

January 21, 2010

Dr. Howell, Ladies and Gentlemen of the Committee,

Thank you for the opportunity to be here today. My name is Annamarie Saarinen. My daughter Eve was diagnosed at 2 days old with a severe mitral valve defect and enlarged heart. She was very nearly sent home – and was in complete heart failure at 5 days old.  24 hours later, she was also diagnosed with SVT and Wolf Parkinson White Syndrome.  In other words, she would never have made her one-week well-baby visit.

During those first weeks, I learned how fragile life is.  I learned that 1in100 babies are born with a heart defect – the world’s most common birth defect – and that nothing I did caused us to become that 1in100.

I also learned to rely on a small monitor that stayed wrapped around Eve’s foot.  It was a pulse oximeter – measuring the oxygenation in her blood.  The phrase “what are her sats today” became part of our vernacular.  Above 95%, we’re good.  Below, her heart wasn’t working the way it needed to.

While most textbooks identify nearly 40 different heart defects, Dr. Jack Rychik, at CHOP says there are probably more than 100 different defects.  Many congenital heart problems are very difficult to identify by fetal or neonatal ultrasound. These issues are even more relevant in hospitals outside major medical centers or in rural areas.

A recent study showed that less than 1/3 of CHDs are diagnosed prenatally.  That leaves 2/3 that are NOT.  Of these, data indicates that routine newborn exams fail to detect at least half (50%) of CHD cases.*

Murmurs often indicate a heart defect, but many serious defects do not present with a murmur immediately after birth. Even with a murmur and a careful exam, additional measures can help increase early detection of life-threatening cardiac disease.

Pulse oximetry – done at the appropriate interval between 24-48 hours after birth – can indeed detect otherwise “silent” heart defects. More than 60% of defects can result in mild desaturation (like Eve’s) not detectable with the eye, but readily detected by pulse oximetry. Simply put, pulse oximetry DOES increase detection of true CHD over exam alone. And the earlier CHD is detected and treated, the more likely a child will survive and have fewer developmental delays and long term health complications.** A baby coming back to the hospital in heart distress is PROVEN to have increased chance of death and a worse neurological outcome that those diagnosed before discharge.

False positive rates have already proven to be quite low – about 1/3 of one percent (.034).  New generation pulse oximeters may result in even greater specificity and fewer false-positives…in addition to catching any number of other respiratory and lung issues that can also go undiagnosed.

I believe the nomination process will clearly show that postnatal screening to identify CHD should be a vital part of normal newborn care.  There are many fine institutions in this country that already realize this and are screening without mandate, including Regions Hospital in Saint Paul, Mary Bridge Pediatric Heart Center in Tacoma, and Children’s National Medical Center right here in Washington DC.

Pulse oximetry testing is non-invasive, easily performed, inexpensive, and requires little training of nursery personnel – even we have one at home. It can simply be done in conjunction with other in-the-nursery screening, such as hearing…and will require very few unnecessary echocardiograms.

I understand the role of this committee in ensuring that suitable newborn screening tests are developed and safe, effective treatments are available for implementation.

Congenital heart disease accounts for the majority of deaths from congenital defects in childhood – six times more common than chromosomal abnormalities.

Nearly twice as many children die from Congenital Heart Defects in the United States each year as from all forms of childhood cancers combined

By any standard, this is a public health need.

I am very respectful of the need for fiscal responsibility in health care  – but have little patience for more babies struggling or dying needlessly.  In the past 3 months alone, I personally know several families who have buried their babies due to undiagnosed heart defects.

Eve’s heart had about a week left when we flew her to Boston for open heart surgery 9 months ago.  Today, she is proof that medical professionals can work their magic on babies with severe heart issues – BUT ONLY when they are given the timely opportunity to do so.  I look forward to the opportunity to keep you informed as our own Minnesota study moves forward supporting the other clinical data pointing to the efficacy of pulse ox screening.  On behalf of the 40,000 US families whose babies will be diagnosed with heart disease this year – and the 4,000 who will not see their first birthdays, I sincerely thank you for your commitment to the health of newborns and children.

~Annamarie Saarinen, 1in100

———————————————–

*Although many of the missed cases will be only a ventriculoseptal 2 defect (VSD), an additional study showed that only 38% of patients with IAA, HLHS, COA, and aortic stenosis (AS) became symptomatic or were clinically diagnosed prior to discharge.7 Some types of CHD have complete mixing of venous and arterial blood, with resulting pulse oximeter readings (SpO2) in the low 90s, and many observers cannot recognize arterial desaturation until the SpO2 drops into the mid- to low-80s. Therefore, mild desaturation may indicate serious CHD and not be recognized.

** March of Dimes

Our heart community took some major hits this week…we lost some very, very precious babies here on earth.  But the angels in heaven gained some new playmates.  I think many of us take the greatest solace in the messages from those parents who have already experienced loss.  Levi Beers (Aiden’s dad) never fails to put the faith back in one’s soul. http://mysonisanangel.wordpress.com/   I am grateful for his counsel and friendship.

And there are so many more…Cora’s mom, Kaia’s mom, James’ mom, Allie’s mom…

Despite their still-fresh grief, they reach out to CHD families and GIVE.  I saw a post from Stephanie, Kaia’s mom, that said she would be sure to have Kaia look for Hazel and Pierce in heaven…show them around, ya know.  Just that visual image provided such comfort and peace.  I have yet to see anything more tragic than a coffin the size of a shoe box.  It’s wrong in every way.  But hearing the hope directly from these parents…that is pure light.

And in the middle of all the darkness this week – there has actually been a good deal of light.

There were two things that I started working on last summer – after Eve’s surgery.  Newborn screening for heart defects.  And pediatric heart devices for surgeons.

Those two things became the cornerstone priorities of 1in100.  There is clearly so much to do in the wide world of CHD.  But for the moment, these were the things I truly thought weren’t getting enough traction.  And that I might have some of the tools to tackle.

So this week – strangely enough, BOTH of these priorities saw major breakthroughs.

1in100 Priority 1 – Newborn Screening/Early Detection

This coming week, for the first time in memory, there will be an official report on the agenda at the national Newborn Screening Advisory Committee meeting in Washington DC.  It will be presented by the Mayo Clinic doctor that is on the Minnesota committee and that has been advising on our pulse ox pilot study here.  I will be providing comments at this meeting – and the next step is to submit the formal nomination to make universal screening for CHD at “standard of care” for all newborns.   Anticipating this will happen as soon as February.

On a related note, there are at least two CHD screening bills at the state level that have been introduced in recent weeks.  Cora’s Law and Chloe’s Law are both making headway.  Kudos to Kristine http://instructionsarenotincluded.blogspot.com/ and Kelly http://chdbabies.blogspot.com/ for their passion and drive.

1in100 Priority 2 – Pediatric Heart Devices

Six months ago I made the rounds with heart device companies to find out why it’s so damn hard to get pediatric size devices into the hands of surgeons operating on tiny hearts.  Some reasons? FDA restrictions, small market, too much R&D, too long…been keeping at it regardless, even provided testimony at an FDA workshop in the fall.  Fast forward: Email from a major heart valve company. As of this week, they are working to develop a pediatric heart valve!!  Everyone (FDA, Industry and Physicians) have now made commitments to the speedy clinical study and introduction of pediatric valves with proven safety to the market.

Stunned and grateful.  I could have never imagined so many good people would mobilize, so quickly, to move these initiatives forward in such a meaningful way.

February 13, 2010

This Valentine’s Weekend.  Put your heart in the right place.  Where your heart on your sleeve.  And Open Your Eyes.

One hundred global events mark the wind-down of Congenital Heart Defect Awareness Week, reinforcing the crusade against heart disease and heart defects in children as a year-round 24/7 battle.

On Saturday, February 13th – just as CHD Awareness Week 2010 is wrapping up – 100 events will be happening in tandem around the world.

There are no rules – these events are personalized to (and in honor of) advocates everywhere who are the front lines want to share what they know about the world’s most common birth defect.  One in one hundred babies born are afflicted with a congenital heart defect.  Personal stories, Newborn Screening, Medical Advancements, Heart Heroes and Rockstars.  It’s time the world heard what’s happening, what’s being done, and we can do.

Heart defect awareness is 24/7/365.  Every day is a good day to Know more and Do more.

1in100 EVENT ROSTER (updated weekly)

1. Annamarie & Paul Saarinen

In honor of Eve Isley and those who Do More everywhere.

Minneapolis, Minnesota

2. Rachel Boyum

In honor of Maggie Stewart

Lakeville, Minnesota

3. Amber Teater Schmidt

In honor of the 3^rd anniversary of Kyleigh’s Open Heart Surgery

Lexington. KY

4. Kristina Daugherty

In honor of heart warrior Aiden (tricuspid atresia – 6 months old, glenn scheduled for March 2010)

St, Louis, MO

5. Kristin Uchida Collier

In honor of Khloe and everyone dealing with CHDs

Denver, CO

6. Kristi Bono Pena

LOCATION

7. Karla Hider

In honor of angel Isla (dilated cardiomyopathy and mitral valve regurgitation)

Cambridge, United Kingdom

8. Kristine Brite McCormick

In honor of angel Cora

Indianapolis, IN (??)

9. Jessica Twigg

In honor of angel Ethin

Michigan

10. Lisa Roseman Brancato

In honor of

Chicago, IL

11. Michelle Shannon

Omaha, NE/Council Bluffs, IA

12. Amber Whitman

In honor of Addison and Cora

Franklin, IN

13. Isabelle & Ron Ouimette

In honor of Andre

Danville, MA

14. Melissa

INFO
Sacramento, CA

15. Holly

(son – TOF, now 28 months)

Burlington, VT

16. Abby Whaley

In honor of angel Allie

Raleigh, NC

17. Laurel Hubert

In honor of Lyla’s first heart birthday

LOCATION TBD

18. Ann Thompson

In honor of Eve

Emmons, Minnesota

19. Josh & Jenny Madigan

In honor of Maddie and Eve

Rosemount, Minnesota

20. Audra Grunwaldt

In honor of Ben

Souix Falls, South Dakota

21. Grete Lekanger

In honor of angel babies

Oslo, Norway

22. Richard & Carolyn Gray

In honor of hearts everywhere

Carmel, CA

23. Bonnie Burkert

In honor of Eve

Los Angeles, CA

24. Mary Calvano & It’s My Heart Michigan

In honor of Drew, CHD Warriors and Angels

TBD, Michigan

25. Levi & Tracy Beers

In honor of angel Aiden

Denver, CO

26. Tracy Dougherty

Centerville, Iowa

27. Kelli Carlson

In honor of Will

TBD, Rhode Island

28. Doug & Gretchen Kingland

In honor of Eve

Northwood, Iowa

29. Melissa Hingos

In honor of Jack & Eve

Raleigh, NC

30. Rene Saarinen

In honor of Eve

Hibbing, MN

31. Tom Reynolds

In honor of friends fighting CHD

Eagle, CO

32. Holly Anderson

Brooklyn Park, MN

33. Kris Huson

In honor of Eve

Minneapolis, MN

34. Monique Bowersox

In honor or Eve

Rochester, MN

35. Tony Weisshauer

Des Moines, IA

36. Denise Sanders

In honor of Michael

Clearwater, FL

Want to host a 1in100 Event?

Post here – Or email annamarie@1in100.org – Or post on Facebook.com/1in100.

Name, Location, Time of Day, in Honor of

More to come!

This is the most popular day for babies to be born…Wednesdays, I mean.  15.4% more births happen on Wednesday than on the average day.  Got me thinking – my first two children, Jack and Elle, were born on Thursdays.  Baby Eve, a Friday.  Not the first time I’ve fallen out of the statistical norm.  We are 1in100 for Pete’s sake.

So I did the math – and it means that today, 126 babies were born in the US with a heart defect.  A study from a few years back cites that routine newborn examinations STILL fail to detect more than half of babies with heart disease; examination at 6 weeks misses one third.

I personally know 17 children sleeping in intensive care units tonight.  I also know 11 families who have buried their babies in the past 5 months, including baby Cora’s parents.   She went home from the hospital with her mommy – just like half of the 126 other CHD babies born this day.  One month ago today, Cora died nursing in her mothers arms.

While this all seems very, very wrong I choose (partly for sanity, partly for peace) to view it as our window in humanity to make some things right.  Simple, safe newborn screening for the world’s most common birth defect is just a start.  Check it out.  Pass it along.

And welcome to the world, sweet Wednesday babies.

What is Newborn Screening with Pulse Oximetry?

Pulse oximetry monitoring uses a light source and sensor to measure oxygen in the blood.
A soft, wrapped sensor is wrapped around the baby’s foot.
Light passing through the foot measures the amount of oxygen in the blood.
The test is quick (3-5 minutes) and painless. Pulse oximetry monitoring should detect most heart defects.

Why is it important to check babies for heart defects?

If undetected, some congenital heart defects can cause serious or even life-threatening problems. Early detection and early treatment lead to better outcomes.

Why check the blood oxygen level with pulse oximetry?

A low oxygen saturation level may indicate the presence of a heart defect.

What are the benefits of the screening?

Babies are less likely to be sent home with unidentified heart problems – some of which can cause acute, emergency situations or even death.  If identified in the first 24-48 hours of life, medical teams are available for diagnosis and treatment of CHDs. Critical congenital heart defects, requiring immediate treatment or repair, can be performed before discharge from the hospital.

Will screening find all types of heart defects?

No current screening tool exists to detect CHDs 100 percent of the time. Pulse oximetry screening should detect most heart defects (those associated with a low blood oxygen level). However, some heart detects may not be found on screening (those not associated with a low blood oxygen level).

What will happen if a baby has a low blood oxygen level?

The pulse oximetry test will be done again. If the level is still lower than expected, then an echocardiogram (sonogram of the heart) will be done. A pediatric cardiologist will ‘read’ the echocardiogram to check for the presence of a heart defect. If a CHD is found, the pediatric cardiologist will start collaborating on those findings and working on treatment options. Most heart defects can be corrected or improved with surgery, procedures and/or medications.

What are the other signs and symptoms of heart defects parents can watch for?

• Baby tires easily during feeding (falls asleep before feeding finishes)
• Sweating around the head, especially during feeding
• Fast breathing when at rest or sleeping
• Pale or bluish skin color
• Poor weight gain
• Sleeps a lot, not playful or curious for any length of time
• Puffy face, hands and/or feet
• Often irritable, difficult to console

Congenital Heart Defects (CHDs) are defects that are present at birth and affect the structure or function of the heart or vessels.

• Heart defects are the most common birth defect.
• CHDs occur in approximately one of every 100 births.
• About 40,000 babies with CHD are born in the US each year.
• Heart defects are the leading cause of newborn and infant death.
• Although some babies will be diagnosed before birth or at birth, sometimes the diagnosis is not made until days, weeks, months or even years later.